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光感受器边缘蛋白是一种由隐性Stargardt病(ABCR)基因编码的ABC转运蛋白。

The photoreceptor rim protein is an ABC transporter encoded by the gene for recessive Stargardt's disease (ABCR).

作者信息

Azarian S M, Travis G H

机构信息

Department of Psychiatry and Program in Neuroscience, University of Texas Southwestern Medical Center, Dallas 75235-9111, USA.

出版信息

FEBS Lett. 1997 Jun 9;409(2):247-52. doi: 10.1016/s0014-5793(97)00517-6.

Abstract

Rim protein (RmP) is a high-Mr membrane glycoprotein that has been localized to the rims of photoreceptor outer segment discs, but its molecular identity is unknown. Here, we describe the purification of RmP and present the sequence of its mRNA. RmP is a new member of the ATP-binding cassette (ABC) transporter superfamily. We show that RmP is expressed specifically in photoreceptors and predominantly in outer segments. Further, RmP is identical to the protein recently shown to be affected in recessive Stargardt's disease. RmP is the first ABC transporter observed in photoreceptors and may play a role in the photoresponse.

摘要

边缘蛋白(RmP)是一种高分子量的膜糖蛋白,定位于光感受器外段盘膜的边缘,但其分子特性尚不清楚。在此,我们描述了RmP的纯化过程并给出了其mRNA序列。RmP是ATP结合盒(ABC)转运蛋白超家族的新成员。我们发现RmP特异性表达于光感受器,且主要在外段表达。此外,RmP与最近被证明在隐性Stargardt病中受影响的蛋白相同。RmP是在光感受器中观察到的首个ABC转运蛋白,可能在光反应中发挥作用。

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